2022 Scientific Sessions

Title:
Never Too Late; Reincorporation of an Isolated Pulmonary Artery of Ductal Origin

Introduction:
Isolated pulmonary artery of ductal origin (IPADO) is a rare congenital heart defect consisting of a pulmonary artery arising from the ductus arteriosus with a normally branching intrapulmonary tree. Untreated IPADO can result in pulmonary artery atresia, recurrent pulmonary infections, pulmonary hypertension, and CHF. Detection of IPADO requires recanalization of ductus arteriosus or surgical BT shunt followed by reincorporation of aberrant pulmonary artery into the main pulmonary trunk.

Clinical Case:
A 6-year-old presented to urgent care with an illness and a murmur was incidentally discovered. He was referred to a cardiologist who confirmed the murmur and an EKG revealed right ventricular hypertrophy. Initial echocardiogram demonstrated absent left pulmonary artery (LPA). At 7 years old, he presented to interventional cardiology clinic where CTA showed small aortopulmonary collaterals to left intrapulmonary circulation along with minimal flow through left pulmonary veins. Pulmonary vein wedge angiogram identified developed intrapulmonary circulation bilaterally with an atretic left pulmonary artery arising from the ductus arteriosus. The patient’s family chose to proceed with interventional management. The ductus arteriosus was recanalized using total occlusion wire. Follow-up pulmonary vein injection showed patency of the LPA with flow extending into ductus arteriosus and aorta. The ductus was subsequently stented. An angiogram at 8 months post stent insertion showed continued restoration of blood flow. Aortic catheterization was performed at 10 months post stent insertion for stent dilation. At 15 months post recanalization, the left pulmonary artery was surgically incorporated into the main pulmonary trunk. A pericardial roll was sewn to LPA to create a continuity measuring 12mm. LPA pressure was measured to be 28/7 mmHg. CT scan at 4 weeks post-surgical reincorporation showed patency of the LPA as well as growth. At most recent follow-up, the patient is 11 years old, 4 years removed from surgical reincorporation. He has been asymptomatic with no disease flare-ups; however, LPA has undergone hypoplasia. CTA reveals LPA that measures 6.2mm. Nuclear lung perfusion scan demonstrates 20% of total pulmonary arterial circulation through the left pulmonary artery.

Discussion:
IPADO is a serious congenital heart malformation that usually presents asymptomatically in early childhood. If left untreated, IPADO can result in recurrent pulmonary infections, pulmonary hypertension, CHF, and early mortality. This case demonstrates an incidental murmur that was discovered in an otherwise asymptomatic patient. The patient received initial therapy via stenting of the ductus arteriosus followed by successful surgical reincorporation of the left pulmonary artery into the main pulmonary trunk. The approach has resulted in the affected lung receiving 20% of total pulmonary circulation. Furthermore, it has eliminated the risk of IPADO-related complications and early mortality. In summary, a thorough cardiovascular physical exam and early stenting of ductus arteriosus enabled the rescue of a pulmonary artery, allowing for improved quality of life for the patient. This is the oldest known patient with IPADO to undergo successful surgical reincorporation of left pulmonary artery.